【摘要】 <正> 原发性血小板增多症(Essential thrombocythemia,简称ET)亦称原发性出血性血小板增多症,被认为是一种以血小板增多为主的骨髓增殖性疾患,极罕见于儿童。Barnhart等统计,迄至1980年在英文刊物上发表的仅5例,其中完全符合诊断标准的只有2例,加上Barnhart等报告的1例共3例。国内尚未见儿童病例报告。现将我院儿科诊治的一例儿童ET用马利兰治疗前后观察结果报告如下。更多还原

【Abstract】 Essential thrombocythemia (ET) is very rare in children. Until 1980, only 3 cases appeared to fulfill all the criteria for its diagnosis in the literature published in English, and none of it was reported in Chinese.This article reported a twelve-year-old boy who had suffered from ET for ten years. The clinical features were recurrent spontaneous bleeding, hepatospleno-megaly, persistent increase in platelet count, the platelets becoming bizarre and huge, bone marrow demonstrating panmyelosis with predominant megakarocytosis and thrombocytosis, megakarocytes being large with many bizarre and multilobu-lated nuclei. The platelet ahdesive function and aggregation in response to ADP were very poor, and factor 3 failed to release adequately.The patient responded to myleran quite well. The bleeding disappeared rapidly. The treatment was continued for six months and then stopped. At that time, the platelet count was 142000/mm3, platelet function, megakarocytes count and figure returned to normal, platelet ultrastructure returned to normal, too.Bleeding in ET is probably due to dysfunction of platelets, an outcome of ultrastructural abnormality.更多还原